Use

This test is useful for evaluating individuals with Coombs-negative chronic hemolysis. Hexokinase (HK) is the first enzymatic step in glycolysis, converting glucose to glucose 6-phosphate. Hexokinase deficiency is a rare cause of chronic nonspherocytic hemolytic anemia with an autosomal recessive inheritance. Clinically, HK deficiency manifests in early onset anemia with variable severity, ranging from mild to severe. Neurologic impairment may also occur in some patients.